Outcomes of Children With Cardiomyopathy Listed for Transplant: A Multi-institutional Study

Anne I. Dipchand, David C. Naftel, Brian Feingold, Robert Spicer, Delphine Yung, Beth Kaufman, James K. Kirklin, Tina Allain-Rooney, Daphne Hsu

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46 Scopus citations

Abstract

Background: Dilated (DCM), restrictive (RCM), and hypertrophic (HCM) cardiomyopathies (CM) in children have varying clinical courses and therapeutic options. Heart transplantation (HTx) offers a chance for long-term survival; but outcomes after listing have not been well defined. Methods: A multi-institutional registry of 3,147 patients listed for HTx (January 1993-December 2006) was used to compare outcomes of 1,320 children with CM (42%) and 1,827 with non-CM (58%) etiologies. Comparisons were made between sub-groups: 1,098 DCM (83%), 145 RCM (11%), and 77 HCM (6%). Results: CM patients had a waitlist mortality of 17% vs 32% for non-CM patients (p < 0.0001), with no difference between the CM sub-groups. Risk factors were younger age, black race (relative risk [RR], 1.65; p = 0.009), mechanical ventilation (RR, 3.17; p < 0.001), and extracorporeal membrane oxygenation (RR, 2.16; p < 0.001). Ten-year survival after listing was 66% for CM vs 53% for non-CM (p < 0.0001). HCM and RCM patients aged < 1 year at the time of listing had the highest waitlist mortality and the lowest overall survival. CM patients had a better 10-year survival after HTx (68% vs 61%, p < 0.0001). Risk factors for death early after HTx included mechanical ventilation at HTx (RR, 3.07; p < 0.001), longer ischemic time (RR, 1.27; p = 0.01), and earlier era (RR, 1.77; p = 0.002). Late risk factors included black race (RR, 3.01; p < 0.001), HCM or RCM (RR, 1.93; p = 0.007), and older age (RR, 1.9; p < 0.001). Conclusion: Children with CM have a lower waitlist mortality and better survival post-HTx than children with a non-CM diagnosis. DCM patients have the best and HCM or RCM patients aged younger than 1 year have the worst overall outcomes.

Original languageEnglish (US)
Pages (from-to)1312-1321
Number of pages10
JournalJournal of Heart and Lung Transplantation
Volume28
Issue number12
DOIs
StatePublished - Dec 1 2009

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ASJC Scopus subject areas

  • Surgery
  • Pulmonary and Respiratory Medicine
  • Cardiology and Cardiovascular Medicine
  • Transplantation

Cite this

Dipchand, A. I., Naftel, D. C., Feingold, B., Spicer, R., Yung, D., Kaufman, B., Kirklin, J. K., Allain-Rooney, T., & Hsu, D. (2009). Outcomes of Children With Cardiomyopathy Listed for Transplant: A Multi-institutional Study. Journal of Heart and Lung Transplantation, 28(12), 1312-1321. https://doi.org/10.1016/j.healun.2009.05.019